Pub Date : 2026-01-29DOI: 10.1183/13993003.02595-2025
Johannes Burtscher,Katharina Hüfner,Lei Xi,Martin Burtscher
{"title":"Hypoxia conditioning: a reappraisal of its preventive and therapeutic potential.","authors":"Johannes Burtscher,Katharina Hüfner,Lei Xi,Martin Burtscher","doi":"10.1183/13993003.02595-2025","DOIUrl":"https://doi.org/10.1183/13993003.02595-2025","url":null,"abstract":"","PeriodicalId":12265,"journal":{"name":"European Respiratory Journal","volume":"8 1","pages":""},"PeriodicalIF":24.3,"publicationDate":"2026-01-29","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"146072882","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":1,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Pub Date : 2026-01-29DOI: 10.1183/13993003.00026-2026
James D Chalmers,Neil J Bullen,Don D Sin
{"title":"The European Respiratory Journal 2026: towards the golden age of respiratory science?","authors":"James D Chalmers,Neil J Bullen,Don D Sin","doi":"10.1183/13993003.00026-2026","DOIUrl":"https://doi.org/10.1183/13993003.00026-2026","url":null,"abstract":"","PeriodicalId":12265,"journal":{"name":"European Respiratory Journal","volume":"58 1","pages":""},"PeriodicalIF":24.3,"publicationDate":"2026-01-29","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"146073057","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":1,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Pub Date : 2026-01-29DOI: 10.1183/13993003.02397-2025
Mohammad Badran,Rene Cortese,Alex Gileles-Hillel,David Gozal
OSA is a prevalent chronic condition characterized by repetitive upper airway collapse that promotes the occurrence of gas exchange abnormalities reflected as intermittent hypoxia (IH) along with heightened risk for the occurrence of end-organ morbidity. Here, we examine the molecular and cellular mechanisms driving OSA-induced morbidity. We describe the maladaptive responses to chronic IH, including stress programs, primarily driven by bursts of reactive oxygen species (ROS) that overwhelm antioxidant defenses and trigger robust, NF-κB-mediated inflammatory cascades (e.g. TNF-α, IL-6). These responses, strikingly different from the adaptive responses to sustained hypoxia, lead to systemic consequences, including endothelial dysfunction, hypertension, and profound metabolic dysfunction with insulin resistance. Understanding this pathophysiology is complicated by marked cellular and tissue heterogeneity, with different cell populations (e.g. endothelium, adipose tissue or different brain regions) exhibiting divergent, context-dependent responses to IH (i.e. inflammation versus repair). Traditional bulk-tissue analyses and clinical metrics, such as the AHI or hypoxic burden, fail to capture in their entirety this cellular and tissue heterogeneity and the critical kinetics of IH, particularly during reoxygenation. Critical knowledge gaps remain, including the need to standardize IH exposure metrics (capturing cycle frequency, hypoxic depth, and reoxygenation kinetics), integrate circadian context and other OSA-related stressors (e.g. episodic hypercapnia, fragmented sleep), account for key biological modifiers (sex, age, genetic background, comorbidities), and determine the potential reversibility of IH-induced injury. Addressing these gaps will be essential to advance OSA diagnostic and therapeutic approaches. Integrating multi-omics profiling and physiological modeling within standardized IH paradigms offers a pathway toward patient-tailored interventions.
{"title":"Mechanisms underlying end-organ injury in sleep apnea.","authors":"Mohammad Badran,Rene Cortese,Alex Gileles-Hillel,David Gozal","doi":"10.1183/13993003.02397-2025","DOIUrl":"https://doi.org/10.1183/13993003.02397-2025","url":null,"abstract":"OSA is a prevalent chronic condition characterized by repetitive upper airway collapse that promotes the occurrence of gas exchange abnormalities reflected as intermittent hypoxia (IH) along with heightened risk for the occurrence of end-organ morbidity. Here, we examine the molecular and cellular mechanisms driving OSA-induced morbidity. We describe the maladaptive responses to chronic IH, including stress programs, primarily driven by bursts of reactive oxygen species (ROS) that overwhelm antioxidant defenses and trigger robust, NF-κB-mediated inflammatory cascades (e.g. TNF-α, IL-6). These responses, strikingly different from the adaptive responses to sustained hypoxia, lead to systemic consequences, including endothelial dysfunction, hypertension, and profound metabolic dysfunction with insulin resistance. Understanding this pathophysiology is complicated by marked cellular and tissue heterogeneity, with different cell populations (e.g. endothelium, adipose tissue or different brain regions) exhibiting divergent, context-dependent responses to IH (i.e. inflammation versus repair). Traditional bulk-tissue analyses and clinical metrics, such as the AHI or hypoxic burden, fail to capture in their entirety this cellular and tissue heterogeneity and the critical kinetics of IH, particularly during reoxygenation. Critical knowledge gaps remain, including the need to standardize IH exposure metrics (capturing cycle frequency, hypoxic depth, and reoxygenation kinetics), integrate circadian context and other OSA-related stressors (e.g. episodic hypercapnia, fragmented sleep), account for key biological modifiers (sex, age, genetic background, comorbidities), and determine the potential reversibility of IH-induced injury. Addressing these gaps will be essential to advance OSA diagnostic and therapeutic approaches. Integrating multi-omics profiling and physiological modeling within standardized IH paradigms offers a pathway toward patient-tailored interventions.","PeriodicalId":12265,"journal":{"name":"European Respiratory Journal","volume":"3 1","pages":""},"PeriodicalIF":24.3,"publicationDate":"2026-01-29","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"146073132","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":1,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Pub Date : 2026-01-29DOI: 10.1183/13993003.01464-2025
Amira Benattia,Gwenaël Lorillon,Constance de Margerie-Mellon,Laetitia Vercellino,Philippe Bonniaud,Charles-Hugo Marquette,Sylvie Chevret,Abdellatif Tazi
BACKGROUNDThe efficacy of cladribine in treating pulmonary Langerhans cell histiocytosis (PLCH) has been suggested in select case reports. Treatment-related malignancies remain a concern.METHODSIn this phase 2 trial, the efficacy and safety of cladribine was evaluated in symptomatic PLCH patients with airflow obstruction and/or a decrease in lung function within the previous year. Cladribine was administered for 4 monthly cycles combined with infectious prophylaxis. Patients were followed up every 3 months during the first year to assess efficacy and then until 48 months. The primary endpoint was the cumulative incidence of response to treatment at 6 months, defined as ≥10% improvement in forced vital capacity (FVC) and/or forced expiratory volume in 1 s (FEV1), with an increase of at least 200 mL in the absolute value of FEV1. The study was registered with www.CLINICALTRIALSgov: NCT01473797.RESULTSTen patients (6 men; median age, 37 years; IQR [33; 47.5]; 6 current smokers) were included. The cumulative incidence of response to treatment at 6 months was 70% (CI, 28.4-90.4). The response to cladribine was associated with a median decrease of 14.9 points (IQR [10.5; 23.5]) in the Saint George's Respiratory Questionnaire score. At 12 months, 5 patients were still responders. The duration of infection prophylaxis was 11.8 months [IQR 11.7; 29]. One patient died before the 12-month visit. The remaining patients were alive at 48 months. No, malignancies were detected.CONCLUSIONSCladribine improved lung function in half of the patients at one year of follow-up and was well tolerated overall.
{"title":"Efficacy and safety of cladribine in adult pulmonary langerhans cell histiocytosis: a phase II study.","authors":"Amira Benattia,Gwenaël Lorillon,Constance de Margerie-Mellon,Laetitia Vercellino,Philippe Bonniaud,Charles-Hugo Marquette,Sylvie Chevret,Abdellatif Tazi","doi":"10.1183/13993003.01464-2025","DOIUrl":"https://doi.org/10.1183/13993003.01464-2025","url":null,"abstract":"BACKGROUNDThe efficacy of cladribine in treating pulmonary Langerhans cell histiocytosis (PLCH) has been suggested in select case reports. Treatment-related malignancies remain a concern.METHODSIn this phase 2 trial, the efficacy and safety of cladribine was evaluated in symptomatic PLCH patients with airflow obstruction and/or a decrease in lung function within the previous year. Cladribine was administered for 4 monthly cycles combined with infectious prophylaxis. Patients were followed up every 3 months during the first year to assess efficacy and then until 48 months. The primary endpoint was the cumulative incidence of response to treatment at 6 months, defined as ≥10% improvement in forced vital capacity (FVC) and/or forced expiratory volume in 1 s (FEV1), with an increase of at least 200 mL in the absolute value of FEV1. The study was registered with www.CLINICALTRIALSgov: NCT01473797.RESULTSTen patients (6 men; median age, 37 years; IQR [33; 47.5]; 6 current smokers) were included. The cumulative incidence of response to treatment at 6 months was 70% (CI, 28.4-90.4). The response to cladribine was associated with a median decrease of 14.9 points (IQR [10.5; 23.5]) in the Saint George's Respiratory Questionnaire score. At 12 months, 5 patients were still responders. The duration of infection prophylaxis was 11.8 months [IQR 11.7; 29]. One patient died before the 12-month visit. The remaining patients were alive at 48 months. No, malignancies were detected.CONCLUSIONSCladribine improved lung function in half of the patients at one year of follow-up and was well tolerated overall.","PeriodicalId":12265,"journal":{"name":"European Respiratory Journal","volume":"2 1","pages":""},"PeriodicalIF":24.3,"publicationDate":"2026-01-29","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"146072884","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":1,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Pub Date : 2026-01-29DOI: 10.1183/13993003.01540-2025
Margaret W Leigh,Adam J Shapiro,Kunal K Chawla,Milan J Hazucha,David E Brown,Feng-Chang Lin,Li Jiang,Johnny L Carson,Stephanie D Davis,Sharon D Dell,Scott D Sagel,Margaret Rosenfeld,Carlos Milla,Kelli M Sullivan,Maimoona A Zariwala,Thomas W Ferkol,Michael R Knowles,
RATIONALENasal nitric oxide (nNO) measurement during velum closure is a useful test for primary ciliary dyskinesia (PCD) but not feasible in young children. Measurement during tidal breathing is an alternative approach.OBJECTIVESTo define a protocol with age-specific cut-off values for nNO measurements during tidal breathing and evaluate as an adjunctive test for PCD.METHODSA standardized method for nNO measurement during tidal breathing was developed and tested at University of North Carolina in children under 6-years with PCD, asthma, cystic fibrosis, and healty controls. Normative curves were generated as a function of age and generalized estimating equations were used to define age-specific cut-off values. The protocol was subsequently validated in 107 children undergoing evaluation for PCD at eight additional sites.RESULTSIn healthy controls, tidal breathing nNO values increased from birth to age 6-years, but remained low for children with PCD. With sensitivity set at 0.98, cut-off values of nNO increased from 13.9 nL·min-1 at 2-months to 47.7 nL·min-1 at 5-years of age. Receiver operating characteristic curves had high areas for nNO cut-offs under each curve, ranging from 0.98 to 0.99 across the ages represented in the study. Validation testing at participating sites showed tidal breathing nNO accurately identified 93% of the 60 children under 6 years with PCD confirmed by ultrastructural ciliary analysis or genetic testing, with high specificity.CONCLUSIONTidal breathing nNO measurement is a useful adjunctive test to distinguish young children with PCD from healthy controls as young as 2-months using age-specific cut-off nNO values.
{"title":"Tidal Breathing Nasal Nitric Oxide Measurement as a Test for Primary Ciliary Dyskinesia in Young Children.","authors":"Margaret W Leigh,Adam J Shapiro,Kunal K Chawla,Milan J Hazucha,David E Brown,Feng-Chang Lin,Li Jiang,Johnny L Carson,Stephanie D Davis,Sharon D Dell,Scott D Sagel,Margaret Rosenfeld,Carlos Milla,Kelli M Sullivan,Maimoona A Zariwala,Thomas W Ferkol,Michael R Knowles, ","doi":"10.1183/13993003.01540-2025","DOIUrl":"https://doi.org/10.1183/13993003.01540-2025","url":null,"abstract":"RATIONALENasal nitric oxide (nNO) measurement during velum closure is a useful test for primary ciliary dyskinesia (PCD) but not feasible in young children. Measurement during tidal breathing is an alternative approach.OBJECTIVESTo define a protocol with age-specific cut-off values for nNO measurements during tidal breathing and evaluate as an adjunctive test for PCD.METHODSA standardized method for nNO measurement during tidal breathing was developed and tested at University of North Carolina in children under 6-years with PCD, asthma, cystic fibrosis, and healty controls. Normative curves were generated as a function of age and generalized estimating equations were used to define age-specific cut-off values. The protocol was subsequently validated in 107 children undergoing evaluation for PCD at eight additional sites.RESULTSIn healthy controls, tidal breathing nNO values increased from birth to age 6-years, but remained low for children with PCD. With sensitivity set at 0.98, cut-off values of nNO increased from 13.9 nL·min-1 at 2-months to 47.7 nL·min-1 at 5-years of age. Receiver operating characteristic curves had high areas for nNO cut-offs under each curve, ranging from 0.98 to 0.99 across the ages represented in the study. Validation testing at participating sites showed tidal breathing nNO accurately identified 93% of the 60 children under 6 years with PCD confirmed by ultrastructural ciliary analysis or genetic testing, with high specificity.CONCLUSIONTidal breathing nNO measurement is a useful adjunctive test to distinguish young children with PCD from healthy controls as young as 2-months using age-specific cut-off nNO values.","PeriodicalId":12265,"journal":{"name":"European Respiratory Journal","volume":"43 1","pages":""},"PeriodicalIF":24.3,"publicationDate":"2026-01-29","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"146072881","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":1,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Pub Date : 2026-01-29DOI: 10.1183/13993003.e6701-2026
{"title":"ERJ Podcast January 2026: Cannabis and the lung.","authors":"","doi":"10.1183/13993003.e6701-2026","DOIUrl":"https://doi.org/10.1183/13993003.e6701-2026","url":null,"abstract":"","PeriodicalId":12265,"journal":{"name":"European Respiratory Journal","volume":"303 1","pages":""},"PeriodicalIF":24.3,"publicationDate":"2026-01-29","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"146072883","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":1,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Pub Date : 2026-01-22DOI: 10.1183/13993003.01968-2025
Jayanth Kumar Narayana,Sanjay H Chotirmall
{"title":"Reply: Methodological clarifications of AI-powered research trend analytics in bronchiectasis.","authors":"Jayanth Kumar Narayana,Sanjay H Chotirmall","doi":"10.1183/13993003.01968-2025","DOIUrl":"https://doi.org/10.1183/13993003.01968-2025","url":null,"abstract":"","PeriodicalId":12265,"journal":{"name":"European Respiratory Journal","volume":"30 1","pages":""},"PeriodicalIF":24.3,"publicationDate":"2026-01-22","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"146021439","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":1,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Pub Date : 2026-01-22DOI: 10.1183/13993003.01973-2025
Francesco Del Galdo,Lucy Elizabeth Thornton,Peter M George
{"title":"ERS/EULAR clinical practice guidelines for connective tissue disease-associated interstitial lung disease: a multidisciplinary, evidence-based approach to best management.","authors":"Francesco Del Galdo,Lucy Elizabeth Thornton,Peter M George","doi":"10.1183/13993003.01973-2025","DOIUrl":"https://doi.org/10.1183/13993003.01973-2025","url":null,"abstract":"","PeriodicalId":12265,"journal":{"name":"European Respiratory Journal","volume":"8 1","pages":""},"PeriodicalIF":24.3,"publicationDate":"2026-01-22","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"146021508","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":1,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Pub Date : 2026-01-22DOI: 10.1183/13993003.02501-2025
Athiththan Yogeswaran,Nils C Kremer,Khodr Tello
{"title":"Multi-beat right ventricular-pulmonary arterial coupling and survival in pulmonary hypertension: are we missing the right signals?","authors":"Athiththan Yogeswaran,Nils C Kremer,Khodr Tello","doi":"10.1183/13993003.02501-2025","DOIUrl":"https://doi.org/10.1183/13993003.02501-2025","url":null,"abstract":"","PeriodicalId":12265,"journal":{"name":"European Respiratory Journal","volume":"11 1","pages":""},"PeriodicalIF":24.3,"publicationDate":"2026-01-22","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"146021436","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":1,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
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